Multiple intradural extramedullary tumours presenting with paraplegia after trauma

Intraspinal nerve-sheath tumours are generally slow growing and are diagnosed after causing symptoms such as back pain and progressive neurological symptoms. We present a rare example of multiple schwannomas located in the thoracolumbar spine in a previously asymptomatic patient who developed severe neurological deficits after a motor-vehicle accident. The exact mechanism of neurological compromise in this patient remains unclear. Circulatory instability in the early post-traumatic course could have contributed to pathogenesis.     

Lumbar teratoma presenting intradural and extramedullary extension in a neonate.

BACKGROUND CONTEXT: The association of teratomas and spinal malformations such as spina bifida, partial sacral agenesis, hemivertebrae, and diastematomyelia has been described in the literature. Reported cases, however, are mainly presacral or sacrococcygeal with an extremely rare presentation of intradural extension. PURPOSE: A case of lumbar teratoma with an intradural extension and extramedullary component and the clinical outcome following surgical treatment are reported. STUDY DESIGN/SETTING: To our knowledge, among the reported teratoma cases with an intradural extension and extramedullary component, our case has a distinguishing feature regarding the involvement of the lumbar spine. It is also the first case, showing no neurological deficit during the postoperative period. METHODS: A full-term, female infant presented with a 30 x 30 x 10 mm lumbar mass covered with normal skin. The mass contained an irregular, bone-like, hard and mobile material accompanying cystic components. Magnetic resonance imaging revealed a total closure defect of the first and second lumbar laminae and a subcutaneous mass with intradural extension. The lesion was found to penetrate the dural sac through an extended exposure from T12 to L3. There was no firm attachment of the intradural, extramedullary component of the lesion. Total removal of the tumor was achieved. RESULTS: The patient was discharged on day 7 without any neurological deficit or sign of hydrocephalus. The pathological examination showed a benign teratoma containing mature cartilage, muscle, adipose tissue, and glandular tissue. Follow-up at 2 years showed no recurrence or neurological deficit and a normal sphincter tone. Urodynamic evaluation was within normal limits. CONCLUSION: Accompanying a spinal dysraphic state, the mature teratoma in our case may support the idea of a tumor actually arising from a dysraphism and growing outward to produce the mass.     

Thoracolumbar intradural extramedullary bronchiogenic cyst

Summary Intradural extramedullary bronchiogenic cysts are rare findings. All five reported cases were located cervically or upper thoracically. To our knowledge, we describe the first case of an intraspinal bronchiogenic cyst in a thoracolumbar location.We present the case of a 41-year-old patient with a known spina bifida occulta who suffered from a continuous, sharp, and therapy-refractory pain in the left leg. Magnetic resonance imaging of the thoracic and lumbar vertebra revealed an intradural extramedullar mass at T12 to L1 level. After laminectomy T-12 through L-1/L-2 and longitudinal opening of the dura mater, the cystic mass was shown to be attached to the conus medullaris and the cauda equina, and therefore could be removed only partially. Histopathological examination revealed the diagnosis of bronchiogenic cyst. We therefore conclude that intradural extramedullary bronchiogenic cysts may appear also at thoracolumbar levels. Surgical resection can be achieved with good outcome.     

Thoracic intradural extramedullary capillary haemangioma

The authors report a rare case of thoracic intradural extramedullary capillary haemangioma, the clinical presentation of which was similar to any other intraspinal mass. Magnetic resonance imaging does not always distinguish this entity from others, but the presence of enlarged vasculature is often a useful clue. Complete resection is the treatment of choice of this hamartomatous lesion.     

Solitary thoracic intradural extramedullary plasmacytoma

Summary The bodies of the vertebrae are common locations for plasma cell diseases such as multiple myeloma and solitary plasmacytoma. Secondary invasion of the epidural space is infrequent but can cause neurological symptoms. Spinal cord compression due to pure intradural plasma cell infiltration is very rare. The authors report a 25-year-old woman who developed a progressive difficulty in walking due to a solitary spinal dural plasmacytoma. This is the first reported example in the English language literature of a purely intradural spinal plasmacytoma in a patient without other myelomatous lesions. An entirely intradural solitary plasmacytoma has a relatively better prognosis.     

Lumbar intradural extramedullary bronchiogenic cyst

The pathological findings of an intradural and extramedullary cyst at the L1 level of the spinal canal are described in a 28-year-old male who presented with chronic lumbago and progressive weakness and numbness in both lower limbs. Histopathological examination revealed the diagnosis of bronchiogenic cyst. Bronchiogenic cysts in the spinal canal are uncommon, and cysts at the L1 level are extremely rare. Their pathogenesis is still poorly understood. They are thought to be a malformation arising from a split notochordal syndrome. We conclude that intradural extramedullary bronchiogenic cysts may appear also at lumbar levels. Surgical resection can be achieved with good outcome.     

Primary spinal intradural extramedullary cysticercosis

BACKGROUND: Spinal cysticercosis represents an uncommon localization of a common parasitic disease and, in most cases, is associated with intracranial involvement. Once confirmed, the entire neuraxis should be evaluated. CASE DESCRIPTION: We present an unusual case of a 60-year-old male patient with isolated localization of spinal intradural extramedullary cysticercosis. The patient was treated successfully with the combination of surgical excision and orally administered albendanzole. We discuss the treatment options, the diagnostic screening, and the possible route of dissemination. CONCLUSIONS: Because this is a rare but potentially life-threatening disease, clinicians should always take it into consideration in the differential diagnosis of spinal space-occupying lesions.     

Sarcoidosis presenting as an isolated intramedullary tumor

We report a case of isolated intramedullary sarcoidosis. The patient developed progressive signs that indicated a spinal tumor, which were investigated with contrast-enhanced magnetic resonance imaging scans. Magnetic resonance imaging clearly revealed an intramedullary lesion, but the diagnosis of sarcoidosis was made on the pathological analysis of the surgical specimen. Magnetic resonance imaging with contrast enhancement is reported in a histologically proven case of intramedullary sarcoidosis. Only 12 other cases of isolated intramedullary sarcoidosis have been reported. We review and discuss these cases according to their clinical presentation, the segmental location of the granulomas in the spinal cord, preoperative and operative diagnoses, and signs for systemic sarcoidosis. In none of the cases was the diagnosis of intramedullary sarcoidosis made before surgery. We think that surgical therapy for intramedullary lesions is the best way to diagnose rare instances of benign lesions like sarcoidosis and to treat them in an appropriate manner.     

Thalamomesencephalic ossified cavernoma presenting with Holmes' tremor

BACKGROUND: Midbrain cavernoma associated with Holmes' tremor is a rare entity. Although there have been 4 other cases of Holmes' tremor caused by a cavernoma, this is the first case that was cured by surgical removal of the cavernoma. In addition, heavy ossification and Holmes tremor as a clinical presentation are 2 unusual features of the cavernoma. Possible mechanisms of these very rare entities are discussed in relation to the present report and relevant literature is reviewed. CASE DESCRIPTION: We present a case of 60-year-old woman with heavily ossified cavernoma of the thalamomesencephalic junction with neuroimaging and histologic features. The only manifestation was Holmes' tremor. The patient was operated on via posterior interhemispheric approach while in the sitting position. After the arachnoid folds of the quadrigeminal cistern were opened, the thin neural tissue on the surface of the dorsal midbrain was incised and the lesion was visualized and totally removed as a single piece. The tremor was almost completely suppressed. CONCLUSION: Ossified cavernoma is a rare entity but has a characteristic MRI appearance. It should be considered in the differential diagnosis of intracerebral hypointense lesions on both T1- and T2-weighted MR images because they are potentially curable by surgical removal.     

Multifocal intradural extramedullary ependymoma. Case report

In this paper, the authors present the case of a patient with multifocal intradural extramedullary ependymoma, and they review 18 previously reported cases. A 32-year-old man presented to the authors' institution with a 1-month history of partial medullary syndrome. Magnetic resonance imaging of the neuraxis revealed multifocal intradural extramedullary lesions at the bulbomedullary junction and C2-3, T5-11, L-2, L-4, L-5, and sacrum. Histological examination revealed a WHO Grade II ependymoma. The literature survey yielded 18 cases of ependymoma at the same location; none of them were multifocal at presentation. The authors analyzed the epidemiological, clinical, and surgical features of all 19 cases reported to date, including the present case. Patients' ages ranged from 24 to 69 years; 15 patients were women and 4 were men. The time elapsed from symptom onset to diagnosis ranged from 1 month to 8 years. Pain (in 13 patients) and medullary syndrome (in 12) were reported as the initial symptoms (information was not provided for 1 patient). Tumors were predominantly located in the thoracic spine (11), but they also occurred in the cervicothoracic (3), cervical (2), and lumbar (2) spine. The remaining tumor was multifocal. Solitary extramedullary tumors were found intraoperatively in 13 patients; 3 were described as exophytic and 3 as extramedullary with some degree of medullary invasion. Histological examination revealed 9 WHO Grade II tumors, 4 Grade III tumors, and 1 myxopapillary tumor. Data obtained for the remaining cases proved inconclusive. The clinical condition improved in 11 patients, remained stable in 2, and worsened (recurrence or progression) in 6. Of the 4 patients with Grade II tumors who presented with recurrence or neuraxis spreading, 3 had meningeal infiltration or adhesion to the pia mater, which does not rule out the possibility of neoplastic remnants in that area. Intradural extramedullary ependymomas are rare, they predominate in women in the 5th decade of life, and pain is the most frequent initial symptom. The extent of resection and the presence of meningeal infiltration seem to be key determinants of prognosis. The present case is the first intradural extramedullary ependymoma (with the exception of those occurring at the conus medullaris and terminal filum) with multiple lesions at presentation.     

Spinal intradural extramedullary tumors. Personal experience

BACKGROUND: Spinal intradural extramedullary tumors account for 2/3 of all intraspinal neoplasms and are mainly represented by meningiomas and schwannomas, with the former accounting for the 25-46% of all primary intraspinal tumors. Technical advances in imaging technique, magnetic resonance imaging (MRI) and surgical procedures have brought about significant better clinical results in the last 2 decades. Neverthless a small percentage of patients still present poor postoperative outcome mainly related to the duration of clinical history, the severity of preoperative neurological deficits and to some specific anatomo-surgical aspects. METHODS: In an effort to clarify the influence of these factors on patient's outcome, the authors analyze the clinical, surgical and prognostic data of 41 patients with intradural extramedullary spinal tumor surgically treated between January 1990 and December 1999. The follow-up period ranged from 1 to 9 years. The clinical history until admission and treatment was 3-48 months for meningiomas and 1-72 months for schwannomas. RESULTS: Morbidity and mortality rate was 5 and 0% for meningiomas and 0 and 6% for schwannomas. Almost all the patients experienced a significant neurological improvement after surgery, with a percentage of Nurick's grade 1 and 2 of 68% among patients with meningiomas and 66% among patients with schwannomas. Removal of the tumor was complete in 90% of meningiomas and 94% of schwannomas. CONCLUSIONS: The authors address radical surgery as the ideal goal in these neoplasms, as it gives the patients the best long-terms results, pointing out the importance of systematic early identification of any main radiculomedullary artery during debulking and dissection of the tumor to avoid any risk of severe postoperative neurological worsening of the patients.     

椎管内髓外硬膜下肿瘤手术治疗的临床分析

[目的]探讨椎管内髓外硬膜下肿瘤的手术治疗结果,分析影响髓外硬膜下肿瘤术前症状与预后及复发的因素。[方法]自2004年1月～2009年1月采用外科手术治疗髓外硬膜下肿瘤84例,男41例,女43例。年龄14～76岁,平均44.2岁。病程1周～15年,平均25.3个月。通过视觉模拟评分(VSA)评估疼痛,Nurick′s分级评估神经功能来探讨手术的疗效,同时调查肿瘤病理诊断类型,术前症状持续时间及肿瘤在髓外硬膜下的位置并分析这些因素对术前神经症状和预后的影响。[结果]随访1～5年,平均30个月。在所有病例中VSA评分从8.0±1.2减到1.2±0.8(P0.005),Nurick’s分级从3.0±1.3减到1.0±0.0(P0.001),患者的术前症状有明显改善。术前症状与肿瘤类型、症状持续时间和肿瘤位置无明显相关性,术后疗效与术前症状严重性及病程长短有关。5例髓外硬膜下肿瘤复发。[结论]髓外硬膜下肿瘤多可通过后路手术切除,大部分病例症状明显改善。积极手术切除是髓外硬膜下肿瘤治疗的有效途径。     

Cervical intradural disc herniation

STUDY DESIGN: A case report of anterior en bloc resected cervical intradural disc herniation and a review of the literature. OBJECTIVE: To discuss the pathogenesis of cervical intradural disc herniation. SUMMARY OF BACKGROUND DATA: Including this study case, only 17 cases of cervical intradural disc herniation have been reported. There have been few detailed reports concerning the pathogenesis of cervical intradural disc herniation. METHODS: A cervical intradural disc herniation at C6-C7, with localized hypertrophy and segmentally ossified posterior longitudinal ligament, is reported in a 45-year-old man who had Brown-Sequard syndrome diagnosed on neurologic examination. Neuroradiologic, operative, and histologic findings, particularly the pathology of the anterior en bloc resected posterior vertebral portion of C6 and C7, were evaluated for discussion of the pathogenesis. RESULTS: Adhesion of dura mater and hypertrophic posterior longitudinal ligament was observed around a perforated portion of the herniated disc, and histologic study showed irregularity in fiber alignment accompanied by scattered inflammatory cell infiltration and hypertrophy in the posterior longitudinal ligament. The cervical intradural disc herniation was removed successfully and followed by C5-Th1 anterior interbody fusion with fibular strut graft. Neurologic recovery was complete except for minor residual sensory disturbance in the leg 7 years after the surgery. CONCLUSIONS: Cervical intradural disc herniation is an extremely rare condition. The pathogenesis remains obscure. Only 16 cases have been reported in the literature, and there has been little discussion concerning the local pathology of the herniated portion. The pathogenesis of the disease in the patient reported here was considered to be the adhesion and fragility of dura mater and posterior longitudinal ligament. This was caused by hypertrophy, with chronic inflammation and ossification of the posterior longitudinal ligament sustaining chronic mechanical irritation to the dura mater, leading to perforation of the herniated disc by an accidental force.     

Concomitant cervical and lumbar intradural intramedullary lipoma

BACKGROUND: Lipomas of the spinal cord are often a component of spinal dysraphic states. Nondysraphic intramedullary spinal cord lipomas are rare, and concomitant isolated cervical and lumbar intradural intramedullary lipomata are very rare. One patient with concomitant isolated nondysraphic cervical and lumbar spinal cord lipomata has been reported and management options discussed. CASE DESCRIPTION: A young girl presented with insidious-onset diffuse neck pain and early myelopathic signs. Conventional radiographs were normal. Magnetic resonance imaging of the whole neuraxis revealed concomitant cervical and lumbar intradural intramedullary lipoma. Subtotal resection of the lesion was performed at both levels, after which the patient improved symptomatically and was ambulant independently. CONCLUSION: Concomitant intradural lipomas at 2 different locations unassociated with a dysraphic state is very rare. Magnetic resonance imaging with fat suppression study is the investigation of choice. Adequate decompression with subtotal removal is the treatment of choice.     

Spinal intradural extramedullary haemangioma: MRI and neurosurgical findings

Summary Haemangiomas, have rarely been encountered in the spinal intradural extramedullary space and the MRI findings of this entity have been described only in a few cases. We present the Magnetic Resonance Imaging (MRI) and surgical findings of a rare case of intradural extramedullary cavernous angioma located at the T1–T2 level in a 65-year-old man presented progressive paraparesis and upper thoracic back pain. On MRI, a well-circumscribed intradural solid mass, 1 cm in diameter, was detected and another enhancing nodular mass was found at the nerve roots of the cauda equina. The thoracic spinal lesion was removed and the histological diagnosis confirmed cavernous haemangioma. Although very uncommon, haemangioma should be included in the differential diagnosis when a spinal intradural extramedullary lesion is discovered and some neuroradiological findings could allow a presumptive diagnosis.     

Primary spinal intradural extramedullary hydatid cyst in a child

BACKGROUND/OBJECTIVE: Spinal hydatid cyst is a serious form of hydatid disease affecting less than 1% of the total cases of hydatid disease. We present a case of pathologically confirmed primary intradural spinal cyst hydatid in an otherwise healthy patient who showed no other evidence of systemic hydatid cyst disease. CASE REPORT: An 8-year-old boy presented with back pain, left leg pain, and difficulty in walking. The patient had no other signs of systemic hydatid cyst disease. An intradural extramedullary cystic lesion was identified with magnetic resonance imaging and was shown to be a hydatid cyst by histopathologic examination after the surgical removal. CONCLUSION: Although extremely rare, primary intradural extramedullary hydatid cyst pathology might be the cause of leg pain and gait disturbance in children living in endemic areas.